In primary sclerosing cholangitis (PSC), the bile ducts inside and outside
the liver become inflamed and scarred. As the scarring increases, the ducts
become blocked. The ducts are important because they carry bile out of the
liver. Bile is a liquid that helps break down fat in food. If the ducts are
blocked, bile builds up in the liver and damages liver cells. Eventually, PSC
can cause liver failure.
Researchers do not know what causes PSC. Among the theories under investigation
are the possible role of bacteria, viruses, and immune system problems. PSC
appears to be associated with ulcerative colitis, a type of inflammatory bowel
The disease usually begins between ages 30 and 60, but the disease can also
arise during childhood. PSC is more common in men than women. PSC progresses
slowly, so a personcan have the disease for years before symptoms develop.
The main symptoms are itching, fatigue, and jaundice, which causes yellowing
of the eyes or skin. An infection in the bile ducts can cause chills and fever.
PSC is diagnosed through cholangiography, which involves injecting dye into
the bile ducts and taking an x ray. Cholangiography can be performed as an
endoscopic procedure (endoscopic retrograde cholangiopancreatography, ERCP),
through radiology or surgery, or with magnetic resonance imaging (MRI) scans.
Treatment includes medication to relieve itching, antibiotics to treat infections,
and vitamin supplements, as people with PSC are often deficient in vitamins
A, D, and K. In some cases, surgery to open major blockages in the common bile
duct is also necessary. Liver transplantation may be an option if the liver
begins to fail.
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American Liver Foundation
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